Predictors of recurrence in Sydenham’s chorea: Clinical observation from a single center

Gürkaş, Esra; Karalök, Zeynep Selen; Taskin, Birce Dilge; Aydoğmuş, Ümmü; Güven, Alev; Değerli̇yurt, Aydan; Bektaş, Ömer; Yılmaz, Cahide

doi:10.1016/j.braindev.2016.04.010

Cited by 35 publications

(37 citation statements)

References 29 publications

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“…These reappearances of chorea were not predictable by either prior rheumatic fever activity or cardiac findings. In contrast, a retrospective study of 90 SC patients in Turkey showed that those adherent to their secondary prophylaxis regimen had a significantly lower recurrence rate (nine out of 82 patients) than those who had only irregular prophylaxis (five out of eight patients) 5. In another study, penicillin adherence rates in all patients with SC were 71% (61 out of 85) and 53% (nine out of 17) in patients with recurrent chorea and adequate follow up information 60.…”

Section: Resultsmentioning

confidence: 84%

“…Classically, SC is expected to resolve in 1–6 months. A retrospective study of 90 patients showed complete remission of motor symptoms in 85% by 6 months, and an additional 5% had complete remission by 1 year 5. In contrast, other groups have reported a greater persistence of motor symptoms in their populations.…”

Section: Introductionmentioning

confidence: 95%

“…No predictive demographic features for a prolonged course were identified. Additionally, recurrences of chorea are not uncommon, occurring in 15–40% of patients,5,7 Identified triggers for relapses have included poor prophylactic penicillin adherence,5 the use of oral contraceptive agents, and pregnancy 8. Investigative laboratory studies assist in eliminating alternative causes of chorea, but do not confirm the diagnosis.…”

Section: Introductionmentioning

confidence: 99%

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Treatment of Sydenham’s Chorea: A Review of the Current Evidence

Dean

Singer

2017

Tremor and Other Hyperkinetic Movements

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Section: Resultsmentioning

confidence: 84%

Section: Introductionmentioning

confidence: 95%

Section: Introductionmentioning

confidence: 99%

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Treatment of Sydenham’s Chorea: A Review of the Current Evidence

Dean

Singer

2017

Tremor and Other Hyperkinetic Movements

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“…132 SC is often self-limiting, albeit after many months or a few years, but it can recur. 133 Pediatric autoimmune disorders are covered in a separate article in this journal.…”

Section: Tremormentioning

confidence: 99%

“…In addition, other agents such as tocilizumab (Il-6 inhibitor) and bortezomib (plasma cell depletor) have been used in isolated refractory NMDAR encephalitis cases. [132][133][134][135] Another exciting new therapeutic option in early-phase experimentation may be ephrin B2, which has been administered to mice together with NMDAR antibody-positive CSF with a resultant reduction in memory and behavior disruption by the NMDAR antibodies. 29,49,101,135,136…”

Section: Treatment Of Autoimmune Movement Disordersmentioning

confidence: 99%

Autoimmune Movement Disorders

Fearon

O’Toole

2018

Semin Neurol

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Autoimmune movement disorders are rare but potentially treatable entities. They can present with an excess or paucity of movement and may have other associated neurological symptoms. These disorders were originally recognized by their classic clinical presentations and the cancers associated with them. Recent emphasis has been targeted on associated, and sometimes causative, antibodies. Although some disorders have stereotypical presentations, the spectrum of abnormalities reported in association with antibodies is widening. Determining whether antibodies are incidental or pathogenic and, hence, foregoing or commencing immunotherapy treatment can be challenging for practicing neurologists. Physicians often have to make the decision to empirically treat patients while awaiting test results. Due to the lack of randomized controlled trials, the ideal immunotherapy treatments and regimens are unknown. Patients with intracellularly targeted antibodies tend to fare less well, while those with extracellularly targeted antibody disorders often respond to treatments reducing antibody production. This review aims to summarize reported adult-onset autoimmune movement disorders to date, and to provide a template for the workup and treatment of suspected disorders. Rarer antibodies that are not yet fully characterized, or reported in a few cases only, will not be covered in detail as these are not likely to be readily commercially available. Childhood disorders will be only be mentioned briefly in the discussion, as there is a separate article in this issue on autoimmune neurologic diseases in children.

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Treatments and Outcomes Among Patients with Sydenham Chorea

Eyre,

Thomas,

Ferrarin

et al. 2024

JAMA Netw Open

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ImportanceSydenham chorea is the most common acquired chorea of childhood worldwide; however, treatment is limited by a lack of high-quality evidence.ObjectivesTo evaluate historical changes in the clinical characteristics of Sydenham chorea and identify clinical and treatment factors at disease onset associated with chorea duration, relapsing disease course, and functional outcome.Data SourcesThe systematic search for this meta-analysis was conducted in PubMed, Embase, CINAHL, Cochrane Library, and LILACS databases and registers of clinical trials from inception to November 1, 2022 (search terms: [Sydenham OR Sydenham’s OR rheumatic OR minor] AND chorea).Study SelectionPublished articles that included patients with a final diagnosis of Sydenham chorea (in selected languages).Data Extraction and SynthesisThis study followed the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guideline. Individual patient data on clinical characteristics, treatments, chorea duration, relapse, and final outcome were extracted. Data from patients in the modern era (1945 through 2022) were entered into multivariable models and stratified by corticosteroid duration for survival analysis of chorea duration.Main Outcomes and MeasuresThe planned study outcomes were chorea duration at onset, monophasic course (absence of relapse after ≥24 months), and functional outcome (poor: modified Rankin Scale score 2-6 or persisting chorea, psychiatric, or behavioral symptoms at final follow-up after ≥6 months; good: modified Rankin Scale score 0-1 and no chorea, psychiatric, or behavioral symptoms at final follow-up).ResultsIn total, 1479 patients were included (from 307 articles), 1325 since 1945 (median [IQR] age at onset, 10 [8-13] years; 875 of 1272 female [68.8%]). Immunotherapy was associated with shorter chorea duration (hazard ratio for chorea resolution, 1.51 [95% CI, 1.05-2.19]; P = .03). The median chorea duration in patients receiving 1 or more months of corticosteroids was 1.2 months (95% CI, 1.2-2.0) vs 2.8 months (95% CI, 2.0-3.0) for patients receiving none (P = .004). Treatment factors associated with monophasic disease course were antibiotics (odds ratio [OR] for relapse, 0.28 [95% CI, 0.09-0.85]; P = .02), corticosteroids (OR, 0.32 [95% CI, 0.15-0.67]; P = .003), and sodium valproate (OR, 0.33 [95% CI, 0.15-0.71]; P = .004). Patients receiving at least 1 month of corticosteroids had significantly lower odds of relapsing course (OR, 0.10 [95% CI, 0.04-0.25]; P &lt; .001). No treatment factor was associated with good functional outcome.Conclusions and RelevanceIn this meta-analysis of treatments and outcomes in patients with Sydenham chorea, immunotherapy, in particular corticosteroid treatment, was associated with faster resolution of chorea. Antibiotics, corticosteroids and sodium valproate were associated with a monophasic disease course. This synthesis of retrospective data should support the development of evidence-based treatment guidelines for patients with Sydenham chorea.

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Predictors of recurrence in Sydenham’s chorea: Clinical observation from a single center

Cited by 35 publications

References 29 publications

Treatment of Sydenham’s Chorea: A Review of the Current Evidence

Treatment of Sydenham’s Chorea: A Review of the Current Evidence

Autoimmune Movement Disorders

Treatments and Outcomes Among Patients with Sydenham Chorea

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